本帖最后由 pathology 于 2016-9-29 12:30 编辑
本周病例(2016.8.28—2016.9.11)
1、你考虑什么诊断?
(右乳)Rosai-Dorfman病(Rosai-Dorfman disease)
Rosai-Dorfman disease, also called sinus histiocytosis with massive lymphadenopathy, is a disorder of massive lymph node involvement that may also involve extranodal sites. It often presents with systemic symptoms (fever, leukocytosis, anemia), and most commonly involves the skin, subcutis / soft tissue, upper respiratory tract, bone, eye or CNS. Cases in the breast are rare.
Grossly, there are circumscribed, often multinodular masses in the breast stroma, which typically do not involve the dermis or subcutis. Histologically, large histiocytes are present with abundant pale eosinophilic cytoplasm and mildly atypical round vesicular nuclei. The background shows lymphocytophagocytosis (empe**olesis) in a background of mature lymphocytes and plasma cells. The histiocytes are immunoreactive for S100 and CD68 and negative for CD1a.
Rosai-Dorfman disease usually resolves spontaneously, although systemic cases may cause death. Recent reports suggest that a subset of Rosai-Dorfman disease exhibit features of IgG4-related disease.
2、需要做什么鉴别诊断?
肉芽肿性乳腺炎、IgG4相关性疾病、Langerhans细胞组织细胞增生症、Erdhemi-chester病、特殊感染、纤维组织细胞瘤、炎性肌纤维母细胞瘤等
3、本病预后如何?
结外Rosai-Dorfman病是一种良性组织细胞持续增生性病变,预后较好。临床上应与淋巴瘤、Langerhans组织细胞增生症等相鉴别。Rosai-Dorfman病尚无系统的治疗方法,可应用抗生素、类固醇激素、外科治疗、放疗和化疗等综合治疗,对重要部位的病变侵犯,彻底的手术切除是最有效方法,术后应跟踪随访。
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本期病例比较典型,结合临床病史、组织学形态,辅以免疫组化则不难诊断。综合回答思路,fusuyong、派瑞松加一分!
希望大家一如既往的支持病理每周一题, |